Myasthenia Gravis
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  • ||||||||||  Journal:  Reliability and Validity of Turkish Myasthenia Gravis-Activities of Daily Living Scale. (Pubmed Central) -  Oct 30, 2021   
    MG-ADL-T had fair internal consistency (Cronbach's α = .67), excellent test-retest reliability (ICC = 0.96) and moderate construct validity (MG-QoL, r = 0.59; QMGS, r = .58; MGC, r = .68). MG-ADL, a unique scale that evaluates activities of daily living (ADL), has good test-retest reliability and construct validity in Turkish MG patients.
  • ||||||||||  Journal, Checkpoint inhibition:  Neuro-ophthalmic Complications of Immune-Checkpoint Inhibitors. (Pubmed Central) -  Oct 30, 2021   
    In this review article, we discuss the most common immune-related adverse events (irAEs) pertaining to the central and peripheral nervous systems and their potential afferent and efferent neuro-ophthalmic manifestations. Early recognition and treatment of these irAEs, and discontinuation of the offending ICI are all critical steps to prevent morbidity and mortality.
  • ||||||||||  Biomarker, Journal:  Serum Biomarkers in Neuro-Ophthalmology: When to Test. (Pubmed Central) -  Oct 30, 2021   
    We aimed to provide an update on the current knowledge surrounding and use of relevant autoimmune biomarkers by correlating specific clinical neuro-ophthalmic manifestations with autoantibody biomarkers. The utility of select biomarkers for myasthenia gravis, neuromyelitis optica spectrum disorder, myelin oligodendrocyte glycoprotein-IgG-associated disorder, opsoclonus-myoclonus syndrome, anti-collapsin-response mediator protein-5 optic neuropathy, and glial fibrillary acidic protein-IgG-associated disease are discussed with particular focus on the clinical contexts in which to consider testing.
  • ||||||||||  dalazatide (ShK-186) / Kineta, KPI Therap
    Review, Journal:  Discovery of K 1.3 ion channel inhibitors: Medicinal chemistry approaches and challenges. (Pubmed Central) -  Oct 30, 2021   
    Selectivity of dalatazide (ShK-186), a synthetic derivate of the sea anemone toxin ShK, was achieved by chemical modification and has successfully reached clinical trials as a potential therapeutic for treating autoimmune diseases...Some small-molecule inhibitors with well-defined structure-activity relationships have been optimized for selective delivery to mitochondria, and these offer therapeutic potential for the treatment of cancers. This overview of K 1.3 inhibitors and methodologies is designed to provide a good starting point for drug discovery to identify novel effective K 1.3 modulators against this target in the future.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Review, Journal:  Short- and Long-Lived Autoantibody-Secreting Cells in Autoimmune Neurological Disorders. (Pubmed Central) -  Oct 30, 2021   
    In myasthenia gravis with predominantly IgG4 autoantibodies against muscle-specific kinase (MuSK), it has been observed that one-time CD20 B cell depletion with rituximab commonly leads to long-term remission and a marked reduction in autoantibody titer, pointing to a short-lived nature of autoantibody-secreting cells...Moreover, presence of GC-like structures in the thymus of myasthenic patients with predominantly IgG1 autoantibodies against the acetylcholine receptor and in ovarian teratomas of autoimmune encephalitis patients with predominantly IgG1 autoantibodies against the N-methyl-d-aspartate receptor (NMDAR) confers increased the ability to generate LLPCs. Here, we review available information on the short-and long-lived nature of ASCs in IgG1 and IgG4 autoantibody-mediated neurological disorders and highlight common mechanisms as well as differences, all of which can inform therapeutic strategies and personalized medical approaches.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Clinical, Review, Journal:  MuSK Myasthenia Gravis Presenting with Bilateral Vocal Cord Abduction Paresis: A Case Report and Literature Review. (Pubmed Central) -  Oct 30, 2021   
    MuSK myasthenia gravis cases can be more refractory to treatment with cholinesterase inhibitors and are more likely to cause exacerbations and myasthenia crisis. Therefore, early and accurate diagnosis with appropriate antibody testing is imperative to avoid delays in treatment to prevent potentially life-threatening outcomes.
  • ||||||||||  Review, Journal:  Perioperative management of myasthenia gravis. (Pubmed Central) -  Oct 30, 2021   
    Due to the heterogeneous morphological changes of the thymus that we found in this study, we can hypothesize that thymus is involved in the pathogenic mechanism of MG with anti-AChR-antibodies and concomitant HT development. No abstract available
  • ||||||||||  Clinical, Journal:  Autophagy-deficiency in bone marrow mononuclear cells from patients with myasthenia gravis: A possible mechanism of pathogenesis. (Pubmed Central) -  Oct 29, 2021   
    Furthermore, defective BM-MNCs differentiation, proliferation and apoptosis were observed due to dysfunctional autophagy. These findings suggest for the first time that BM-MNCs autophagy is impaired in patients with MG before immunomodulation therapy, and that autophagy is indispensable for the survival of BM-MNCs, implicating autophagy might be a potential pathogenic mechanism of MG and a novel therapeutic strategy for MG treatment.
  • ||||||||||  Journal:  Anaesthetic Implications of Psychotropic and Neurologic Agents (Pubmed Central) -  Oct 28, 2021   
    Perioperative withdrawal of specific medication implies the risk of recurrence of the neurological symptoms. Therefore, these drugs should be continued postoperatively as soon as possible.
  • ||||||||||  Familial coaggregation of idiopathic inflammatory myopathies and other autoimmune diseases () -  Oct 28, 2021 - Abstract #SCR2021SCR_298;    
    Our findings confirm familial autoimmunity in patients with IIM. We also add knowledge on which and what extent other autoimmune diseases in first-degree relatives are associated with IIM, information that may be useful for clinical counselling and guiding future research on the genetics of IIM.
  • ||||||||||  Clinical, Journal:  COVID-19 Vaccination in Patients with Myasthenia Gravis: A Single-Center Case Series. (Pubmed Central) -  Oct 28, 2021   
    Twenty (90.9%) patients did not present MG symptom worsening within 4 weeks of COVID-19 vaccination, and two (9.1%) patients reported slight symptom worsening but resolved quickly within a few days. Our findings suggest inactivated COVID-19 vaccines might be safe in MG patients with Myasthenia Gravis Foundation of America (MGFA) classification I to II, supporting the recommendation to promote vaccination for MG patients during the still expanding COVID-19 pandemic.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Clinical, Journal:  Immunotherapy choice and maintenance for generalized myasthenia gravis in China. (Pubmed Central) -  Oct 28, 2021   
    Our findings suggest inactivated COVID-19 vaccines might be safe in MG patients with Myasthenia Gravis Foundation of America (MGFA) classification I to II, supporting the recommendation to promote vaccination for MG patients during the still expanding COVID-19 pandemic. Rituximab as monotherapy and tacrolimus with corticosteroids displayed better clinical efficacy as well as drug maintenance to prevent disease relapses in patients with generalized MG.
  • ||||||||||  Journal:  Immunoactive treatment for myasthenia gravis; a Chinese experience. (Pubmed Central) -  Oct 28, 2021   
    Rituximab as monotherapy and tacrolimus with corticosteroids displayed better clinical efficacy as well as drug maintenance to prevent disease relapses in patients with generalized MG. No abstract available
  • ||||||||||  Journal:  Myasthenia gravis relapses: effect of stress, depression and personality (Pubmed Central) -  Oct 27, 2021   
    Both patients benefited from treatment with 3,4-diaminopyridine, a reversible blocker of voltage gated potassium channels at the nerve terminal that prolongs the action potential and increases acetylcholine release. No abstract available
  • ||||||||||  New trial:  Immune Profiles in Myasthenia Gravis (clinicaltrials.gov) -  Oct 26, 2021   
    P=N/A,  N=163, Not yet recruiting, 
  • ||||||||||  Review, Journal:  New Challenges Resulting From the Loss of Function of Na1.4 in Neuromuscular Diseases. (Pubmed Central) -  Oct 25, 2021   
    Developing therapeutic tools able to counteract Na1.4 LoF in skeletal muscles is then a new challenge in the field of Na channelopathies. Here, we review the current knowledge regarding Na1.4 LoF and discuss the possible therapeutic strategies to be developed in order to improve muscle force in SCW.
  • ||||||||||  Clinical, Journal:  The Lived Experience of Myasthenia Gravis: A Patient-Led Analysis. (Pubmed Central) -  Oct 24, 2021   
    Here, we review the current knowledge regarding Na1.4 LoF and discuss the possible therapeutic strategies to be developed in order to improve muscle force in SCW. This patient-driven analysis enriches our understanding of the reality of living with MG from the patient perspective.
  • ||||||||||  Review, Journal:  Neuromuscular Disease Affecting the Larynx. (Pubmed Central) -  Oct 22, 2021   
    These diseases include myasthenia gravis or certain specified neuromuscular junction disorders, primary disorders of muscle, and secondary myopathies. The following discussion selects NMDs from each group, which may have unique laryngeal features and more commonly involve the laryngologist and highlights different diagnostic and treatment considerations related to the larynx.
  • ||||||||||  Journal:  The changing spectrum of drug-induced myopathies. (Pubmed Central) -  Oct 22, 2021   
    More recently, since the introduction of the immune checkpoint inhibitors for the treatment of advanced malignancies, it has been increasingly recognised that these drugs also have a propensity to induce or exacerbate a variety of immune-mediated myopathies, neuropathies, myasthenic disorders and atypical overlap syndromes, and it is anticipated that these complications will become even more prevalent with increasing use of these medications in the future. This review focusses mainly on these two groups of drugs, and on cytokine-based therapies and VEGF inhibitors which have also been implicated in the induction of immune-mediated inflammatory myopathies.
  • ||||||||||  Journal:  Giant cell myositis and myocarditis revisited. (Pubmed Central) -  Oct 22, 2021   
    As suspicion is the key approach in diagnosis, the physician following patients with thymoma with or without myasthenia gravis and with orbital myositis should always be alert. The fatal nature of GCMc associated with these relatively benign diseases deserves a special emergency attention with prompt institution of combined immunosuppressive treatment and very early inclusion of heart failure teams.
  • ||||||||||  Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    Journal:  Myasthenia gravis: MuSK MG, late-onset MG and ocular MG. (Pubmed Central) -  Oct 22, 2021   
    Low dose corticosteroids are usually sufficient. Thyroid disease is the most common autoimmune disease accompanying all three subgroups.
  • ||||||||||  dimethyl fumarate / Generic mfg.
    [VIRTUAL] Isolated Cranial Nerve Palsies in Multiple Sclerosis () -  Oct 21, 2021 - Abstract #ANA2021ANA_477;    
    Isolated cranial nerve palsies are rare presentations of MS and even rarer symptoms of relapse as compared to new onset internulcear ophthalmoplegia and optic neuritis which are more common ophthalmic presen tations of MS. Zadro et al.
  • ||||||||||  mycophenolate mofetil / Generic mfg.
    [VIRTUAL] Myositis and Myocarditis Associated with PD-1 Inhibitor () -  Oct 21, 2021 - Abstract #ANA2021ANA_471;    
    This case is consistent with other reported cases of PD-1 inhibitor induced myositis and highlights the spectrum of neuromuscular side-effects that can be seen. As the use of immune check point inhibitors grows, continued awareness of immune related adverse events by neurologist will be key for early recognition and treatment.
  • ||||||||||  [VIRTUAL] Geographical Variation in Proportion of Musk Antibody Myasthenia Gravis Around the World - A Multicenter Study () -  Oct 21, 2021 - Abstract #ANA2021ANA_444;    
    Overall, as in many previous studies, MuSK Ab patients were younger, had more severe disease and required additional therapies to achieve good outcomes than those patients who were AChR/MuSK antibody negative. There are limitations in the variable number of samples from each center, and less than perfect global distribution of the centers, but these intriguing and unexpected observations suggest that, although MuSK-antibodies are associated with similar disease around the world, environmental factors, including climate, as well as possible ethnic/genetic factors may predispose to this form of myasthenia gravis.
  • ||||||||||  propranolol / Generic mfg.
    [VIRTUAL] Unilateral Persistent Ptosis and Opthalmoplegia and Elevated Antiganglioside Antibodies in a Patient with Myasthenia Gravis () -  Oct 21, 2021 - Abstract #ANA2021ANA_437;    
    He was diagnosed with Grave’s disease in 2019 and was treated with Methimazole and propranolol which improved his muscle weakness partially...Our case is unique in the fact that our patient is Seropositive for both acetylcho line antibodies (blocking and binding antibodies) along with Ganglioside antibodies (anti GM1 and anti GD1b) which to our knowledge was never reported. Elevated anti-ganglioside antibodies in patients with NMJ may contribute to atypical manifestation of NMJ disease, in this case, persistent unilat eral ophthalmoplegia and ptosis
  • ||||||||||  [VIRTUAL] Late Onset Myasthenia Gravis Mimicking Acute Stroke () -  Oct 21, 2021 - Abstract #ANA2021ANA_432;    
    When prescribing Eculizumab, physi cians should obtain adequate sexual histories from the patients and educate them on safe sexual practices. After cerebrovascular events have been ruled out, late-onset Myasthenia Gravis should be in the differen tial diagnosis of sudden onset dysarthria in the elderly population.